Biliary tract anomalies in thoraco-omphalopagus conjoined twins

Thoraco-omphalopagus conjoined twins were separated urgently on the ninth day of life because of bowel obstruction and impending intestinal perforation. Preoperative studies clarified the cardiac, intestinal, and hepatic parenchymal anomalies, but the precise biliary anatomy remained obscure. Operative cholangiogram through the single gallbladder visualized only the extrahepatic biliary anatomy in twin A. At reoperation for abdominal patch removal, exploration in twin A found biliary atresia. Because of the unusual duodenal anatomy and extreme short bowel, the authors elected to treat the biliary atresia in twin A with an appendix portoenterostomy. Twin B had no gallbladder and a profoundly hypoplastic biliary tree. Both twins have normal bilirubin and liver function at 18 months of age. The anatomic details of 8 additional cases of complex biliary tract anomalies in thoraco-omphalopagus conjoined twins are reviewed. In all cases, the complex biliary anatomy has been associated with conjoined duodenum. In most cases the preoperative technetium 99m HIDA scan has been inconclusive or misleading.